Peripheral Nervous System Disease in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study

Research output: Contribution to journalArticle


Objective: To determine the frequency, clinical characteristics, associations, and outcomes of different types of peripheral nervous system (PNS) disease in a multiethnic/multiracial, prospective inception cohort of systemic lupus erythematosus (SLE) patients. 

Methods: Patients were evaluated annually for 19 neuropsychiatric (NP) events including 7 types of PNS disease. SLE disease activity, organ damage, autoantibodies, and patient and physician assessment of outcome were measured. Time to event and linear regressions were used as appropriate. 

Results: Of 1,827 SLE patients, 88.8% were female, and 48.8% were white. The mean ± SD age was 35.1 ± 13.3 years, disease duration at enrollment was 5.6 ± 4.2 months, and follow-up was 7.6 ± 4.6 years. There were 161 PNS events in 139 (7.6%) of 1,827 patients. The predominant events were peripheral neuropathy (66 of 161 [41.0%]), mononeuropathy (44 of 161 [27.3%]), and cranial neuropathy (39 of 161 [24.2%]), and the majority were attributed to SLE. Multivariate Cox regressions suggested longer time to resolution in patients with a history of neuropathy, older age at SLE diagnosis, higher SLE Disease Activity Index 2000 scores, and for peripheral neuropathy versus other neuropathies. Neuropathy was associated with significantly lower Short Form 36 (SF-36) physical and mental component summary scores versus no NP events. According to physician assessment, the majority of neuropathies resolved or improved over time, which was associated with improvements in SF-36 summary scores for peripheral neuropathy and mononeuropathy. 

Conclusion: PNS disease is an important component of total NPSLE and has a significant negative impact on health-related quality of life. The outcome is favorable for most patients, but our findings indicate that several factors are associated with longer time to resolution.


  • John G. Hanly
  • Qiuju Li
  • Li Su
  • Murray B. Urowitz
  • Caroline Gordon
  • Sang Cheol Bae
  • Juanita Romero-Diaz
  • Jorge Sanchez-Guerrero
  • Sasha Bernatsky
  • Ann E. Clarke
  • Daniel J. Wallace
  • David A. Isenberg
  • Anisur Rahman
  • Joan T. Merrill
  • Paul R. Fortin
  • Dafna D. Gladman
  • Ian N. Bruce
  • Michelle Petri
  • Ellen M. Ginzler
  • M A Dooley
  • Kristjan Steinsson
  • Rosalind Ramsey-Goldman
  • Asad A. Zoma
  • Susan Manzi
  • Munther A. Khamashta
  • Graciela S. Alarcón
  • Elisabet Svenungsson
  • Ronald F. van Vollenhoven
  • Cynthia Aranow
  • Meggan Mackay
  • Guillermo Ruiz-Irastorza
  • Manuel Ramos-Casals
  • Sung Sam Lim
  • Murat Inanc
  • Kenneth C. Kalunian
  • Soren Jacobsen
  • Christine A. Peschken
  • Diane L. Kamen
  • Anca Askanase
  • Chris Theriault
  • Vernon Farewell
External organisations
  • Dalhousie University
  • University of Cambridge
  • University of Toronto
  • University of Birmingham
  • Hanyang University
  • Salvador Zubirán National Institute of Health Sciences and Nutrition
  • McGill University
  • University of Calgary
  • University of California, Los Angeles
  • University College London
  • Oklahoma Medical Research Foundation
  • Laval University
  • University of Manchester
  • Johns Hopkins University School of Medicine
  • State University of New York Downstate Medical Center
  • University of North Carolina
  • National University Hospital of Iceland
  • Northwestern University Feinberg School of Medicine
  • Hairmyres Hospital
  • Allegheny Health Network
  • King's College London
  • University of Alabama
  • Karolinska University Hospital
  • Academic Medical Center of University of Amsterdam (AMC)
  • Feinstein Institute for Medical Research
  • University of the Basque Country
  • Hospital Clínic of Barcelona
  • Emory University
  • Istanbul University
  • University of California, San Diego
  • Copenhagen University Hospital
  • University of Manitoba
  • Medical University of South Carolina
  • NYU Langone
  • Toronto Western Hospital
  • Instituto Nacional de Ciencias Médicas y Nutrición
  • Cedars-Sinai Medical Center
  • St Thomas' Hospital
  • Karolinska Institutet
  • Institutd' Investigacions Biomèdiques August Pi iSunyer (IDIBAPS)
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Rheumatology and Autoimmunity
Original languageEnglish
Pages (from-to)67-77
JournalArthritis and Rheumatology
Issue number1
Early online date2019 Aug 7
Publication statusPublished - 2020
Publication categoryResearch